Signs and symptoms method in neuropsychology: A preliminary investigation of a standardized clinical interview for assessment of cognitive decline in dementia.

Psychologists usually perform a preliminary assessment of the person's cognitive status through a brief interview conducted before the formal testing. However, this exam has not yet been standardized with ad hoc recommendations in psychology literature. In this work, a standard observational NeuroPsychological Examination (NPE) designed for psychologists was proposed, and its clinical effectiveness evaluated. The NPE was administered to patients referred to a neuropsychological service in a memory clinic over a 2-year period. The NPEs of the patients with Alzheimer dementia (AD), vascular dementia (VaD), and healthy controls (HC) were retrospectively retrieved. Comparisons among the three groups were conducted. Abnormalities/signs identified during the NPE in the AD and VaD groups are more numerous compared to those reported in the HC group. About 80% of HCs show none or only one abnormal sign. Vice versa, 87.5% of both AD and VaD patients show three or more abnormalities. Accordingly, the NPE has 0.88 (95%CI = 0.81-0.95) sensitivity and 0.95 (95%CI = 0.88-1.02) specificity for detecting cognitive decline when a cut-point of three or more signs is applied. Some significant differences also emerge on the number of pathological signs between AD and VaD patients. NPE is a promising tool with demonstrated diagnostic utility in dementia patients.

Does the Right Focal Variant of Alzheimer's Disease Really Exist? A Literature Analysis.

BACKGROUND: Alzheimer's disease (AD) is a clinically heterogeneous disease. Multiple atypical syndromes, distinct from the usual amnesic phenotype, have been described. In this context, the existence of a right variant of AD (RAD), characterized by enduring visuospatial impairment associated with right-sided asymmetric brain damage, has been proposed. However, to date, this phenotype remains controversial. In particular, its peculiar characteristics and the independence from more prevalent cases (especially the posterior cortical atrophy syndrome) have to be demonstrated. OBJECTIVE: To explore the existence of focal RAD on the basis of existing literature. METHODS: We performed a literature search for the description of atypical AD presentations, potentially evoking cases of focal RAD. To be considered as affected by RAD, the described cases had to present: 1) well documented right-sided asymmetry at neuroimaging; 2) predominant cognitive deficits localizable on the right hemisphere; 3) no specific diagnosis of a known variant of AD. RESULTS: Twenty-one cases were found in the literature, but some of them were subsequently excluded because some features of a different clinical syndrome were overlapped with the clinical features of RAD. Thirteen positive cases, three of them with pathologically confirmed AD, remained. A common right clinical-radiological syndrome, characterized by memory and visuospatial impairment with temporal and parietal involvement, consistently emerged. However, the heterogeneity among the reports prevented a definitive and univocal description of the syndrome. CONCLUSION: Even if sporadic observations strongly support the existence of a focal RAD, no definitive conclusions can still be drawn about it as an independent condition.

Familial late-onset Alzheimer's disease: description of an Italian family with four affected siblings and one case of early-onset dementia in the preceding generation.

We describe a family composed of six siblings, four of which affected by late-onset Alzheimer's disease (LOAD). We constructed the family pedigree, evaluated mutations usually associated with early-onset Alzheimer's disease (APP, PSEN1, PSEN2), and assessed polymorphisms in the apolipoprotein E (APOE) gene and in cytokine genes that we had previously found to be associated with a higher risk of LOAD (IL-10, IL-6, TNF-α). Results showed that all subjects carried one ε4 allele of the APOE gene and those with the earliest age of onset exhibited the AA (-1082) IL-10 and the CC (-174) IL-6 genotypes. The only male had a genetic profile which also included the A (-308) TNF-α allele. These data confirm the role of the APOE gene as genetic risk factor in LOAD, and suggest that the risk of developing AD may be governed by a "susceptibility profile" involving polymorphisms in inflammatory genes.

Preclinical polymodal hallucinations for 13 years before dementia with Lewy bodies.

OBJECTIVE: We describe a case of dementia with Lewy bodies (DLB) that presented long-lasting preclinical complex polymodal hallucinations. BACKGROUND: Few studies have deeply investigated the characteristics of hallucinations in DLB, especially in the preclinical phase. Moreover, the clinical phenotype of mild cognitive impairment-(MCI-) DLB is poorly understood. METHODS: The patient was followed for 4 years and a selective phenomenological and cognitive study was performed at the predementia stage. RESULTS: The phenomenological study showed the presence of hypnagogic and hypnopompic hallucinations that allowed us to make a differential diagnosis between DLB and Charles Bonnet syndrome (CBS). The neuropsychological evaluation showed a multiple domain without amnesia MCI subtype with prefrontal dysexecutive, visuoperceptual, and visuospatial impairments and simultanagnosia, which has not previously been reported in MCI-DLB. CONCLUSIONS: This study extends the prognostic value of hallucinations for DLB to the preclinical phases. It supports and refines the MCI-DLB concept and identifies simultanagnosia as a possible early cognitive marker. Finally, it confirms an association between hallucinations and visuoperceptual impairments at an intermediate stage of the disease course and strongly supports the hypothesis that hallucinations in the earliest stages of DLB may reflect a narcolepsy-like REM-sleep disorder.